先天性肺囊性腺瘤样畸形的诊断和治疗

The diagnosis and treatment of congenital cystic adenomatoid malformation of the lung in children

目的 总结小儿先天性肺囊性腺瘤样畸形的诊断及手术治疗 ,提高对该病的认识。方法 对 2 4例术前均摄胸部X线平片、CT并经手术、病理证实的先天性肺囊性腺瘤样畸形病儿的临床资料进行回顾性总结。其中男 13例 ,女 11例 ,平均年龄 4 75岁 ,主要症状是呼吸道感染及呼吸困难 ,病史 1d至 8年。结果 影像学术前确诊率 79 17%。 2 4例行开胸术 ,证实病变均为单侧 ,侵犯 1个肺叶 2 2例 ,其中左叶 15例、右叶 7例 ;侵犯 2个以上肺叶者 2例。 14例与支气管无交通 ,1例合并胸主动脉的异常血供 ,1例合并巨大实性肿物。 2 0例行肺叶切除、3例行肺段切除、1例因胸膜肺母细胞瘤行全肺切除。全组无术后并发症。按Stocker分型 :I型 13例 ,II型 9例 ,III型 2例。结论 影像学检查是诊断先天性肺囊性腺瘤样畸形的主要方法。对有症状者 ,手术切除病变侵犯的肺组织是治疗该病安全、有效的方法。

Objective: To summarize the experience of diagnosis and treatment of congenital cystic adenomatoid malformation (CCAM) of the lung in children. Methods: 24 patients with CCAM were surgical treated. There were 13 males and 11 females. The mean age was 4.75 years. The symptoms were recurrent pulmonary infection and respiratory distress from 1 day to eight years. Preoperative chest films and CT scan were required in all cases and were confirmed at thoracotomy and pathology. Results: The preoperative diagnosis rate of image examination approach to 79.17%. 24 patients underwent a thoracotomy. The malformation was unilateral in all patients. The involvement of single lobe was identical in 22 cases (left 15, right 7). Lesions involved more than one lobe in 2 patients. 14 cases were lack of communication with brobchi; One had an aberrant pulmonary artery vessel coming from thoracic aorta;One accompanied with a huge solid mass. 20 cases received lobectomy, 3 segmentectomy and 1 pneumonectomy for accompanying with pleuro-pulmonary blastoma. There were no postoperative complications presented in this series. The histopathology was classified according to Stocker: 13 type I, 9 type II and 2 type III. Conclusion: Imaging examination is strongly required to evaluate CCAM. It is the effective way to pulmonary resections in symptomatic patients.